Ioana Preston, MD - phaware® interview 518

Dr. Ioana Preston explores the emerging field of pulmonary hypertension associated with interstitial lung disease (PH-ILD). She discusses the growing recognition of PH-ILD, especially after the introduction of inhaled treprostinil as a treatment, and highlights the importance of early screening and diagnosis. Dr. Preston also delves into the challenges of treating rare diseases, the evolution of research, and the hope for future therapies that could significantly improve patients' lives. This insightful conversation sheds light on a critical yet often overlooked aspect of pulmonary care, urging clinicians to stay vigilant and informed about PH-ILD's complexities.

This Special Edition episode is sponsored by Liquidia.

I'm Ioana Preston. I'm a physician and the pulmonary hypertension expert. I'm the Pulmonary Hypertension Director at Lahey Hospital and Medical Center in Burlington, Massachusetts.

Today, I would like to talk to you about a rare form of pulmonary hypertension -- pulmonary hypertension associated with interstitial lung disease or ILD. PH-ILD is typically a disease of an adult. There is a small population of children, but it’s kind of a different entity. It's typically an adult disease because mostly adults develop interstitial lung disease.

If we look at PAH Group 1, there are more women than men with a Group 1 PAH. This ratio does not translate in PH-ILD, where the population is more mixed. A typical PH-ILD patient is adult or elderly. They could be men or women. It's much broader in presentation. It's not a very specific population that we need to look at. That's very important, because we have to keep our mind open to screen all patients who have ILD for pulmonary hypertension.

Now, at the 7th World Symposium on Pulmonary Hypertension that just took place in summer of 2024 renamed this entity and called it ILD-PH. So, interstitial lung disease with PH associated. Now, we haven't talked about this entity for a long time until recently. Why haven't we talked about it? Because there were no therapies available. We were not looking for pulmonary hypertension development in patients with ILD such as pulmonary fibrosis. It was kind of in the back of our minds as clinicians. But because there were no therapies available, we couldn't have any impact on the well-being of our patients with ILD who may develop pulmonary hypertension.

Now, recently, after many, many years and many, many trials that failed, couple of years ago, a Phase 3 clinical trial showed that inhaled treprostinil is efficacious in treating pulmonary hypertension associated with interstitial lung disease. At that point, the door was opened and we clinicians understood that we need to look for this development in patients with ILD. We need to recognize it early. We need to screen for the presence of pulmonary hypertension in ILD. We need to treat it if it is available.

Moreover, I think what is also important is to help the community develop other therapies for PH-ILD, because the story just begins. It doesn't end here. So, developing other therapies by enrolling patients with PH-ILD in clinical trials, I think it's a very important aspect that we need to focus on in the next years.

So, going back to PH-ILD, what it means is the development of high blood pressure in the lungs or pulmonary hypertension in patients who have underlying forms of, or different types of pulmonary fibrosis. Clinicians now need to focus and learn how to screen and identify this entity. This educational program that occurred at CHEST 2024 is focused on teaching physicians and clinicians and healthcare providers how often and what tests are required to look for pulmonary hypertension, how to diagnose it, how to understand the severity of the pulmonary hypertension and how to treat it. So, now we have a better understanding and a more clear plan of how to approach pulmonary hypertension in the setting of interstitial lung disease.

Going back 30 years ago, when I was a young physician in training, at that time, we had only one treatment for pulmonary arterial hypertension, which is Group 1 PAH. We had only Flolan. At that time, what was happening is we would diagnose patients with PAH and then we would wait for the disease to become severe, and then we would start therapy, because it was an infusion therapy. But this first drug that was developed in PAH opened a huge opportunity in research and clinical practice in PAH. Now, we have 14 therapies.

Now, fast-forward in the 2020s, the same is happening. The same story is being repeated with PH-ILD. Up until very recently, all the clinical trials have failed, so we had no therapy for PH-ILD. Now, we have one therapy, so the door has opened. I am very hopeful that we will have more and more therapies in the future, in the next years to come to offer our patients to better control their disease, to improve their symptoms, and hopefully to have an impact on their life. That's why I'm really hopeful that we will have more options for our patients in the future.

So, now fast-forward medical history to the 2020s, for PH-ILD there is a wealth of clinical research in clinical trials that are looking at different drugs and compounds to add on to the current therapies that we have available. There's very interesting research. I'm very hopeful that in the very near future, we will have more opportunities to treat PH-ILD effectively and improve the breathing, the activity, and the lifestyle of patients with PH-ILD.

The rare diseases are challenging in and out of itself. The more rare the disease, the less experience the clinician has. We are humans and we think of more common things more often as a cause of our patient's symptoms. We forget the rare conditions, lack of experience, the fact that we in general clinical practice, not in my clinic, but in general clinical practice, you don't tend to see many patients with pulmonary fibrosis or ILD. Therefore, it's difficult to remember the subsets of comorbidities or other conditions that an ILD patient can develop.

I think the first step is the fact that there's a rare disease, it's way in the back of the clinician’s mind. That's why it's very important to increase awareness of rare diseases such as PH-ILD. It's very important to remind clinicians to not forget about this entity and also to provide patients with pulmonary fibrosis information about this possible occurrence during their lifetime.

But I have to say, if you look at different reports, I remember one report from Virginia from Inova Fairfax. It’s a large lung transplant center, and there patients with ILD get lung transplants. So, there is a strict requirement to look for pulmonary hypertension at the beginning of lung transplant evaluation, and also to evaluate for pulmonary hypertension just before they have the transplant. This report showed that at the time of transplant, 84% of patients with ILD just before they go to the OR, have pulmonary hypertension.

So, we are talking about pulmonary fibrosis and all sorts of interstitial lung diseases. They're not common, they're rare. However, as the disease progresses, the vast majority of these patients will develop pulmonary hypertension. So, we really have to be cognizant of this important occurrence.

My name is Dr. Ioana Preston, and I'm aware that my patients are rare.

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